Courtesy of the British Sjögren’s Syndrome Association.
Sjögren’s Syndrome is the UK’s second most common autoimmune rheumatic disease, yet the condition remains under-recognised and frequently under-treated. It does not command a high profile within the medical profession, and the general public is largely unaware of the problems faced by sufferers. In reality, Sjögren’s Syndrome is a debilitating, distressing and miserable condition.
- It affects approximately 3-4% of adults in the UK, with a mean age of 50 years.
- 90% of patients are women.
In Sjögren’s Syndrome the secretary glands that produce saliva, tears, vaginal and other secretions develop a form of inflammation, similar to that seen in the joints in patients with rheumatoid arthritis. As a result of this, these glands stop working, leading to dry eyes, dry mouth, dry skin and also dryness of the gastrointestinal tract. These features, as well as being very uncomfortable and unpleasant, mean those sufferers may have difficulty swallowing dry foods, and also dryness of the large bowel can cause symptoms similar to Irritable Bowel Syndrome (IBS).
The clinical presentation of Sjögren’s Syndrome is variable, but typical symptoms include:
- Dry, itchy, irritated eyes
- Dry mouth and difficulty swallowing
- Joint pain and muscle aches
- Low mood, irritability and impaired concentration
- Disabling fatigue
Sjögren’s Syndrome is an autoimmune disease, a condition where the body’s immune system starts attacking parts of the body instead of fighting infection; this is mainly the white cells, which travel in the blood stream. In the case of Sjögren’s Syndrome, they attack the glands, which produce tears, saliva, skin, bowel and other secretions.
Sjögren’s Syndrome can occur later in the course of other rheumatic conditions, such as rheumatoid arthritis, lupus or scleroderma; this is known as Secondary Sjögren’s Syndrome. Most patients with these conditions will already be under the care of a rheumatologist who will usually make the diagnosis of Sjögren’s Syndrome as soon as the dryness symptoms are mentioned.
It can also occur, however, as a condition in its own right (Primary Sjögren’s Syndrome). It typically develops in women in their 30s to 60s, although it can occur at any age and affects both men and women. Features such as generalized muscle or joint aching and generalized fatigue can be the most prominent symptoms. The fatigue isn’t an ordinary tiredness where you can have a good night’s sleep and wake up feeling better, it’s a really quite overwhelming fatigue. In the situation where a person’s main symptom is tiredness, and particularly if the dryness is not reported, the diagnosis may not be made for a long time, if at all. As a result of this, although the evidence suggests that Sjögren’s Syndrome is as common as rheumatoid arthritis, there is a significant proportion of people in whom it is not being correctly diagnosed and symptoms are put down to ‘old age’ for example, and people are suffering in silence.
A number of studies have shown that the health-related quality of life of patients with Primary Sjögren’s Syndrome is significantly decreased and is comparable with that of patients with a condition like Systemic Lupus Erythematosus (SLE).
Despite being a common disorder, Sjögren’s Syndrome has not commanded a high profile and has been under-diagnosed and many patients have gone untreated, causing unnecessary pain and suffering. There are serious complications for some patients, such as neuropathies, loss of teeth and lymphoma, and permanent damage or ulceration to the eye, which can lead to sight loss. Prompt diagnosis and treatment can prevent these complications from happening.
Similarities between M.E./CFS and Sjögren’s Syndrome are discussed in Norheim KB et al,. A possible genetic association with chronic fatigue in primary Sjögren's syndrome: a candidate gene study, Rheumatol Int, 3 Sep 2013.
These are some of the complications that can affect a sufferer:
- Arthritis, Raynaud’s syndrome and fatigue
- Lung involvement
- Kidney involvement
- Neurological involvement
- MALT Lymphoma (44 times increased risk)
- Patients present to different specialties, due to their varied symptoms
- Often non-specific symptoms
- Long lead time before diagnosis
- Often partial diagnosis only
- Undiagnosed patients not receiving appropriate therapy
- Encourage regular dental review (6 monthly)
- Good dental hygiene (brushing, flossing, fluoride)
- Avoid excess sweet foods
- Avoid strong soaps (use aqueous creams and emollients)
- Avoid dry environments
- Avoid cigarette smoking and excess alcohol
- Receive information about self-help groups (e.g. BSSA)
The British Sjögren’s Syndrome Association (BSSA) was founded in 1986, as a registered charity, to raise awareness of the disease and support research into its cause and treatment. A self-help organisation with more than 1800 members, the BSSA is dedicated to providing mutual support and information to individuals affected by this disabling disease. We have regional support groups throughout the UK, whose members include sufferers and supporters who work together in helping one another cope with the day-to-day challenges of this debilitating and distressing condition. The BSSA also has a helpline, we distribute a variety of literature, including an informative quarterly newsletter, and we award an annual research grant. There is currently no cure for Sjögren’s Syndrome and the cause is still unknown.